کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
3257540 | 1207420 | 2010 | 9 صفحه PDF | دانلود رایگان |
![عکس صفحه اول مقاله: Clinical and immunological overlap between autoimmune lymphoproliferative syndrome and common variable immunodeficiency Clinical and immunological overlap between autoimmune lymphoproliferative syndrome and common variable immunodeficiency](/preview/png/3257540.png)
Autoimmune lymphoproliferative syndrome (ALPS) is mainly caused by defects in the CD95 pathway. Raised CD3+TCRαβ+CD4−CD8− double negative T cells and impaired T cell apoptosis are hallmarks of the disease. In contrast, the B cell compartment has been less well studied. We found an altered distribution of B cell subsets with raised transitional B cells and reduced marginal zone B cells, switched memory B cells and plasma blasts in most of 22 analyzed ALPS patients. Moreover, 5 out of 66 ALPS patients presented with low IgG and susceptibility to infection revealing a significant overlap between ALPS and common variable immunodeficiency (CVID). In patients presenting with lymphoproliferation, cytopenia, hypogammaglobulinemia and impaired B cell differentiation, serum biomarkers were helpful in addition to apoptosis tests for the identification of ALPS patients. Our observations may indicate a role for apoptosis defects in some diseases currently classified as CVID.
Journal: Clinical Immunology - Volume 137, Issue 3, December 2010, Pages 357–365